The antioxidant capacity of erythrocyte concentrates is increased during the first week of storage and correlated with the uric acid level.

BACKGROUND AND OBJECTIVES: Red blood cells (RBCs) suffer from lesions during cold storage, depending in part on their ability to counterbalance oxidative stress by activating their antioxidant defence. The aim of this study was to monitor the antioxidant power (AOP) in erythrocyte concentrates (ECs) during cold storage. MATERIALS AND METHODS: Six ECs were prepared in saline-adenine-glucose-mannitol (SAGM) additive solution and followed during 43 days. The AOP was quantified electrochemically using disposable electrode strips and compared with results obtained from a colorimetric assay. Haematological data, data on haemolysis and the extracellular concentration of uric acid were also recorded. Additionally, a kinetic model was developed to extract quantitative kinetic data on the AOP behaviour. RESULTS: The AOP of total ECs and their extracellular samples attained a maximum after 1 week of storage prior to decaying and reaching a plateau, as shown by the electrochemical measurements. The observed trend was confirmed with a colorimetric assay. Uric acid had a major contribution to the extracellular AOP. Interestingly, the AOP and uric acid levels were linked to the sex of the donors. CONCLUSION: The marked increase in AOP during the first week of storage suggests that RBCs are impacted early by the modification of their environment. The AOP behaviour reflects the changes in metabolism activity following the adjustment of the extracellular uric acid level. Knowing the origin, interdonor variability and the effects of the AOP on the RBCs could be beneficial for the storage quality, which will have to be further studied.

Storage of red blood cell concentrates: Clinical impact.

The storage of red blood cells for transfusion purposes induces modifications of biochemical and biological properties. Moreover, these modifications are modulated by the donors' characteristics and the cell processing. These ex vivo alterations were suspected to decrease the transfusion efficiency and even to induce adverse events. This short article will review the red blood cells storage lesions and the clinical data related to them. In particular, the questions regarding the donors and recipients sex will be discussed.

Irreversible oxidations of platelet proteins after riboflavin-UVB pathogen inactivation.

Pathogen inactivation technologies are known to alter in vitro phenotype and functional properties of platelets. Because pathogen inactivation generates reactive oxygen species, oxidative stress is considered as one of the plausible cause at the origin of the platelet storage lesion acceleration after treatment. To date proteomics has been used to document the protein variations to picture out the impact. Here, platelet concentrates were prepared from buffy-coats in Intersol additive solution, leukoreduced and pathogen inactivated using a riboflavin/UVB treatment. At day 2 of storage the platelet proteomes of control (untreated) and treated platelet concentrates were investigated against the site specific oxidation by liquid chromatography coupled to tandem mass spectrometry in a shotgun experiment. The shotgun approach detected 9350 peptides (and 2534 proteins) of which 1714 were oxidized. Eighteen peptides were found exclusively oxidized in treated platelets whereas 3 peptides were only found oxidized in control. The present data evidenced an interference with several proteins involved in platelet aggregation and platelet shape change (such as talin and vinculin).

Towards the understanding of the UV light, riboflavin and additive solution contributions to the in vitro lesions observed in Mirasol®-treated platelets.

OBJECTIVES: Pathogen reduction technologies are implemented to increase the safety of blood products. We previously showed that the UVB alone significantly contributes to the storage lesions observed in platelets treated with riboflavin/UVB using a home-made illuminator. The present study aims at confirming these observations using the commercial Mirasol® technology. METHODS: A three-arm study (untreated, UV-, Mirasol®-treated platelets) was conducted to investigate the platelet storage lesions throughout storage (n=4). A two-arm study was then designed to compare Intersol and T-PAS+ additive solutions (n=3). Phenotype and functional platelet characteristics were assessed using flow cytometry, aggregometry, antioxidant assays and metabolic parameters. RESULTS: Mirasol®-treated platelets exhibit enhanced storage lesions compared to controls (increase of activation markers and glycolysis rate, lower hypotonic shock and double-agonist activation responses, and decrease of total antioxidant capacity). Here, we also confirmed that the UV radiation alone is causing platelet lesions. Riboflavin tends to have an intracellular protective role while it decreases the extracellular antioxidant defenses. Furthermore, benefits of platelet additive solutions containing potassium and magnesium were confirmed as it reduces the extent of storage lesions. CONCLUSIONS: The photosensitizer, UV illumination and composition of the platelet additive solutions are key parameters influencing the platelet storage lesion. The clinical relevance of these findings is not fully understood and recent published clinical studies could not show increase in bleeding in patients receiving Mirasol-treated platelets. New developments in storage solutions might help to improve storage conditions of PRT-treated platelets and should be prioritised as research subject in the future.

Oxidative stress and antioxidant defenses during blood processing and storage of erythrocyte concentrates.

Oxidative lesions start accumulating in cells when the oxidant attacks overwhelm the antioxidant defenses. This review will briefly describe red blood cell storage lesions with emphasis on the consequences of oxidation and the cellular defense mechanisms, as well as the methods that can be used to monitor them. The sources of variability in red blood cell storage capacity depend on the donor characteristics, the product processing and the storage conditions. Suggestions to improve the product quality in order to ensure the best efficacy and safety for the transfused patient are also discussed.

The storage lesions: From past to future.

Red blood cell (RBC) concentrates are stored in additive solutions at 4oC for up to 42 days, whereas platelets concentrates (PCs) are stored at 22oC with continuous agitation for up to 5 to 7 days, according national regulations, and the use or not of pathogen inactivation procedures. Storage induces cellular lesion and alters either RBC or platelet metabolism, and is associated with protein alterations. Some age-related alterations prove reversible, while other changes are irreversible, notably following protein oxidation. It is likely that any irreversible damage affects the blood component quality and thus the transfusion efficiency. Nevertheless, there still exists a debate surrounding the impact of storage lesions, for both RBCs and PCs. Uncertainty is not completely resolved. Several studies show a tendency for poorer outcomes to occur in patients receiving older blood products; however, no clear significant association has yet been demonstrated. The present short review aims to promote a better understanding of the occurrence of storage lesions, with particular emphasis on biochemical modifications opening discussions of the future advancement of blood transfusion processes. The paper is also an advocacy for the implementation of an independent international organization in charge of planning and controlling clinical studies in transfusion medicine, in order to base transfusion medicine practices both on security principles, but also on clinical evidences.

[Langerhans-cell histiocytosis in twin sisters]. / Histiocytose langerhansienne chez deux soeurs jumelles.

BACKGROUND--Histiocytosis of Langerhans cells includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, Letterer-Siwe syndrome and Hashimoto-Pritzker histiocytosis. These syndromes represent a spectrum of severity and prognosis of the same underlying disorder which is usually sporadic. It has occurred in monozygotic twins and in a familial pattern. This report describes monozygotic twins who developed the disease a few months after their father was found to be suffering from Hodgkin's disease. Case n. 1.--A 4 month-old girl was admitted because of fever, disseminated lymphadenopathy and hepatomegaly. She also had interstitial pneumonia. Infiltrating abnormal histiocytes were demonstrated in lymph node and bone marrow biopsies. X-rays showed lytic areas in the skull. Serology for EBV infection was negative. Special studies with immune markers of lymph node histiocytes confirmed the diagnosis of Langerhans cell histiocytosis, and more precisely, Letterer-Siwe syndrome. The patient was given prednisolone followed by vinblastine without success. She was given etoposide 11 weeks later, which induced remission. This treatment was replaced by vinblastine when the patient was aged 2 years 9 months. Case n. 2.--The monozygotic twin of the case n. 1 was also admitted at 4 months of age because of the same manifestations. Laboratory findings were identical to those of her sister, as was her response to the same drugs. The father was diagnosed as having Hodgkin's disease 3 months before the first manifestation of Langerhans cell histiocytosis in his daughters. His maternal uncle had also been treated for Hodgkin's disease. Immunologic studies of the twin were negative. CONCLUSION--These cases of Langerhans cell histiocytosis in monozygotic twins have no apparent relationship with the Hodgkin's disease of their father. Etoposide seems to be useful for treating such severe forms of the disease.

[Acute basal ganglia necrosis with favorable course during Mycoplasma encepahlitis]. / Nécrose aiguë des noyaux gris d'évolution favorable lors d'une encéphalite à mycoplasme.

BACKGROUND: Acute bilateral striatal necrosis complicating the course of a post-infectious encephalitis is rare. CASE REPORT: A previously healthy 5-year-old boy presented with an atypical pneumonia; he rapidly developed, encephalitis revealed by a generalized status epilepticus. After transient improvement, he became confused and mutic, with dystonic postures of his limbs. Painful stimulation resulted in prolonged facial grimacing and doleful cry. CT scan and MRI showed abnormal signals in the whole basal ganglia, typical of bilateral striatal necrosis. Serologic tests for Mycoplasma pneumoniae were positive. The child recovered almost completely. CONCLUSION: A parainfectious process is probably responsible for the transient bilateral striatal necrosis seen in this patient who had Mycoplasma pneumoniae infection several days before the onset of neurologic symptoms. MRI seemed more reliable than CT-scan for the diagnosis of this condition.

[Proteomics and transfusion medicine]. / Protéomique et médecine transfusionnelle.

The term "proteomics" covers tools and techniques that are used to analyze and characterize complex mixtures of proteins from various biological samples. In this short review, a typical proteomic approach, related to the study of particular and illustrative situation related to transfusion medicine is reported. This "case report" will allow the reader to be familiar with a practical proteomic approach of a real situation, and will permit to describe the tools that are usually used in proteomic labs, and, in a second part, to present various proteomic applications in transfusion medicine.

Improving NGO collaboration in AIDS prevention in rural Haiti.

PIP: Zanmi Lasante (Health Friends), a nongovernmental organization (NGO) based in rural Haiti, provides an example of the effective role NGOs can play in acquired immunodeficiency syndrome (AIDS) prevention. The group has produced a video, "Chache Lavi, Detwi Lavi," based on the true story of a rural woman who became infected when she migrated to Port-au-Prince to seek employment. The video illustrates the association between human immunodeficiency virus (HIV) and broader social issues such as peasants' access to land, political upheaval, and unequal gender relationships. To promote collaboration, Save the Children established the NGO Coalition for the Prevention of AIDS and Sexually Transmitted Diseases (STDs) in the Central Plateau. Women, particularly adolescent females, are the coalition's target population. Activities have included literacy and income generation training for women, development of algorithms for STD treatment without laboratory diagnosis, a training course for health professionals on STD services and counseling, training of trainers workshops, and preparation of informational materials. The 10 participating NGOs and private voluntary organizations meet quarterly to share information and evaluate programs.^ieng

[Clear cell sarcoma of the tendons and aponeuroses]. / Sarcome à cellules claires des tendons et aponévroses.

BACKGROUND: Clear cell sarcoma of tendons and aponeuroses is a rare mesenchymal tumor in childhood. It is difficult to treat and its prognosis is bad. CASE REPORT: A 9 year-old girl was admitted because of persistent pain in the left lower part of her thorax. Clinical examination showed a mass, 4 cm in diameter, embedded in the thoracic wall. Ultrasonography and CT-scan showed that this mass extended between the last ribs to the pleural cul-de-sac. There was no adenopathy and myelogram was normal. Biopsy of the mass showed features of clear cell sarcoma of tendons and aponeuroses. Cytogenetic studies showed translocation t(12;22) (q13-14;q12) in tumor cells. The patient was given chemotherapy followed by complete resection, but the tumor recurred locally 3 months later and the patient died, despite chemotherapy plus radiotherapy, 23 months after the apparent onset of disease. CONCLUSION: Treatment of this type of tumor remains difficult. The existence of a break point on 22q12, as in Ewing sarcoma, suggests that this tumor in of neural crest cell origin.

Minocycline-induced clinical and biological lupus-like disease.

A 14-year-old girl developed maculopapular rash, myalgias, arthralgias and myocarditis with elevated anti-nuclear and anti-double-stranded DNA antibodies. She was taking minocycline for acne and all symptoms resolved when this treatment was stopped. The patient has no evidence of disease one year after onset of symptoms. Clinicians should be aware of minocycline's responsibility in inducing lupus-like disease.

Adrenobronchial fistula complicating a neonatal adrenal abscess: treatment by percutaneous aspiration and antibiotics.

A case of retroperitoneal pulmonary fistula caused by a neonatal adrenal abscess is reported. The adrenal abscess was diagnosed by means of needle aspiration which guided the choice of antibiotic therapy. The fistula was demonstrated by direct injection of contrast medium into the adrenal abscess. Treatment by needle aspiration of the adrenal abscess and intravenous antibiotics was successful.

Diffuse leukodystrophy in an infant with cytochrome-c oxidase deficiency.

A 25-month-old boy, born to consanguineous parents, had progressive spastic tetraplegia, and increased signal of the white matter on cerebral T2-weighted magnetic resonance imaging indicative of diffuse leukodystrophy. Elevated blood and cerebrospinal fluid lactate levels pointed to a respiratory chain defect. Cytochrome-c oxidase deficiency was demonstrated in cultured skin fibroblasts and skeletal muscle. This report extends the phenotype of COX deficiency in infancy. Systematic study of blood and CSF lactate should be carried out in every infant with leukodystrophy.